Pediatric Cleft and Craniofacial Anomalies Center

What is Craniosynostosis?

Diagram of skull suturesCraniosynostosis, or premature closure of the cranial sutures, occurs when the bones in a baby's skull fuse together before the brain has stopped growing. This can happen before the baby is born or in the first few months of life. When these growth plates fuse together prematurely, deformity can occur due to restricting expansion of the skull. When the growth is restricted in a certain direction, the brain will push outward in an alternative direction of least resistance and result in skull deformity. This results in a misshapen skull and face. The characteristic patterns of deformity caused by craniosynostosis are readily recognized by the trained eye, even without imaging studies. Nevertheless, a three dimensional CAT scan is often performed.

Causes of Craniosynostosis

This condition has several possible causes, including:

X-ray of craniosynostosis skull - top view

  • Prenatal restraint of head growth, caused by early engagement of the baby's head into the mother's pelvis, or by sharing the uterus with a twin sibling
  • Genetic syndrome disorder, such as Crouzon or Apert Syndrome
  • Fetal exposure to toxins such as phenytoin, valproic acid, or other teratogens (extremely rare occurrence).

Craniosynostosis can also occur sporadically, with no known cause or related condition. These cases typically involve a single fusion, whereas cases related to syndromes will be more complex, involving the fusion of several sutures, as well as other conditions. In the case of either sporadic or syndromic occurrences, there are no known causes or risk factors.

Treatment for Craniosynostosis

X-ray of craniosynostosis skull - front view

Treatment is aimed at restoring the normal shape and relationship of the forehead, orbital rims, and skull. Without surgical intervention, the skull deformities caused by craniosynostosis persist through life. Adults with untreated craniosynostosis are easily recognizable. While the deformity may vary from very mild to severe, the physical and emotional effects of a noticeably misshapen forehead and face can cause problems and are very important reasons for seeking treatment.

The goal of the modern surgical treatment of craniosynostosis is the definitive and long-term correction of the face and skull deformity. We are fortunate at Golisano Children's Hospital to be able to offer the newest and minimally invasive techniques for repair of craniosynostosis. If your child is diagnosed early enough, endoscopic suture release is possible. This procedure removes the closed-growth plate and allows the brain to expand unrestricted. It is very important that this minimally-invasive procedure be followed by about a year of helmet molding therapy. Sometimes a second helmet is needed as the baby's head rapidly grows. The helmet guides the growth of the brain into its new, rounder shape. The baby will regrow the bone that was removed, usually by the time he or she is a year old.

X-ray of craniostenosis skull - front viewWhat to Expect

Find out what to expect during your child's hospital stay and treatment for craniosynostosis.

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